Plasmapheresis for hemolytic crisis and impending acute liver failure in Wilson disease

2007 ◽  
Vol 22 (5) ◽  
pp. 295-298 ◽  
Author(s):  
Samuel Asfaha ◽  
Mohammed Almansori ◽  
Uwais Qarni ◽  
Klaus S. Gutfreund
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson Disease ◽  
Hemolytic Crisis

Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease

2013 ◽  
Vol 81 (5) ◽  
pp. 498-500 ◽  
Author(s):  
Nishant Verma ◽  
Gautham Pai ◽  
Pankaj Hari ◽  
Rakesh Lodha
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Plasma Exchange ◽  
Wilson Disease ◽  
Hemolytic Crisis

scholarly journals Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis

2021 ◽  
Vol 11 (01) ◽  
pp. e145-e147
Author(s):  
Nida Mirza ◽  
Ravi Bharadwaj ◽  
Smita Malhotra ◽  
Anupam Sibal
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson Disease ◽  
Prognostic Score ◽  
Prognostic Index ◽  
Kidney Injury ◽  
Copper Metabolism ◽  
Life Saving ◽  
The Brain

AbstractWilson disease (WD) is a disorder of copper metabolism resulting in accumulation of copper in vital organs of the human body, predominantly in the liver and the brain. Acute liver failure in WD has a bad prognosis, especially with a score ≥11 in the revised WD prognostic index; emergency liver transplantation is considered the only life-saving option in this scenario. Here, we reported a girl patient with WD-induced liver failure and poor prognostic score who was rescued by plasmapheresis. She also manifested severe Coombs negative hemolytic anemia and acute kidney injury. This case report highlights the utility of an adjunctive modality besides liver transplantation for the management of fulminant liver failure caused by WD.

scholarly journals Wilson disease: more than meets the eye

2018 ◽  
Vol 94 (1112) ◽  
pp. 335.2-347 ◽  
Author(s):  
Claire Kelly ◽  
Marinos Pericleous
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
High Mortality ◽  
Mortality Risk ◽  
Wilson Disease ◽  
Copper Metabolism ◽  
High Mortality Risk ◽  
High Index Of Suspicion

Wilson disease is a rare but important disorder of copper metabolism, with a failure to excrete copper appropriately into bile. It is a multisystem condition with presentations across all branches of medicine. Diagnosis can be difficult and requires a high index of suspicion. It should be considered in unexplained liver disease particularly where neuropsychiatric features are also present. Treatments are available for all stages of disease. A particularly important presentation not to overlook is acute liver failure which carries a high mortality risk and may require urgent liver transplantation. Here, we provide an overview of this complex condition.

scholarly journals Acute Liver Failure due to Wilson Disease: Eight Years of the National Liver Transplant Program in Uruguay

2019 ◽  
Vol 18 (1) ◽  
pp. 187-192 ◽  
Author(s):  
Victoria Mainardi ◽  
Karina Rando ◽  
Marcelo Valverde ◽  
Daniela Olivari ◽  
Jorge Castelli ◽  
...  
Keyword(s):  
Liver Failure ◽  
Liver Transplant ◽  
Acute Liver Failure ◽  
Wilson Disease ◽  
Transplant Program

scholarly journals Aetiology, Clinical Profiles, Laboratory Profile, Outcome and Prognostic Factors of Pediatric Acute Liver Failure: Experience at a Tertiary Hospital of Bangladesh

2019 ◽  
Vol 10 (1) ◽  
pp. 56-61
Author(s):  
Afsana Yasmin ◽  
ASM Bazlul Karim ◽  
Md Rukunuzzaman ◽  
Kamal Hossen ◽  
Luthfun Nahar ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson Disease ◽  
Laboratory Data ◽  
Tertiary Hospital ◽  
Medical College ◽  
Pronostic Factors ◽  
Clinical Profiles ◽  
Pediatric Acute Liver Failure

Introduction: Acute liver failure is one of the common causes of death in pediatric gastroenterology and hepatology department. Outcome is different according to aetiology. Objective: To observe the aetiology, outcome and prognostic factors of pediatric acute liver failure. Methods: Consecutive 62 children aged 2 to 16 years of age who were diagnosed as acute liver failure from November 2015 to April 2018 were included in this study. All the clinical profiles, laboratory data and outcome were recorded in a preformed data sheet. Data were analysed by SPSS for Windows version 20. Results: Mean age was 8.5 years. Thirty-nine (62.9%) patients were between 5-10 years of age. Male were 53%. We made a diagnosis of 39 (63%) patients as Wilson disease alone, Another 3 Wilson disease acute liver failure patients had concomitent with either HAV, HEV or HSV in each one. HAV only was responsible for 17 patients and HEV for 1. One patient was Haemophagocyic lymphohistiocytosis and aetiology could not be identified in 1 patient. The overall death in study population was 48% (30). Twenty-four (57%) of 42 acute liver failure patients due to Wilson disease had died. Five (29%) of 17 patients due to HAV infection and 1 patient with HLH died. Ascites, high total bilirubin, high INR and etiology like Wilson disease were the worse pronostic factors for outcome of acute liver failure in children. Conclusion: Wilson disease was the most common aetiology of acute liver failure in children in this study. Early diagnosis is essential as outcome was worse. Majority of viral etiology improved with supportive care. Anwer Khan Modern Medical College Journal Vol. 10, No. 1: Jan 2019, P 56-61

scholarly journals Methanobactin reverses acute liver failure in a rat model of Wilson disease

2016 ◽  
Vol 126 (7) ◽  
pp. 2721-2735 ◽  
Author(s):  
Josef Lichtmannegger ◽  
Christin Leitzinger ◽  
Ralf Wimmer ◽  
Sabine Schmitt ◽  
Sabine Schulz ◽  
...  
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Rat Model ◽  
Wilson Disease

Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices

2014 ◽  
Vol 29 (2) ◽  
pp. 380-386 ◽  
Author(s):  
Harshad Devarbhavi ◽  
Rajvir Singh ◽  
Channagiri K Adarsh ◽  
Keyur Sheth ◽  
Ravi Kiran ◽  
...  
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson Disease ◽  
Prognostic Indices ◽  
Disease Specific
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